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  • Title: Atlantoaxial Instability Treated with Posterior Atlantoaxial Fixation in Two Siblings with Dyggve-Melchior-Clausen Syndrome.
    Author: Shah A, Bhambere S, Ranjan S, Dandpat S, Goel A.
    Journal: Turk Neurosurg; 2020; 30(6):956-960. PubMed ID: 33216345.
    Abstract:
    AIM: To discuss the rare association of atlantoaxial instability in patients with the Dyggve-Melchior-Clausen syndrome, a rare autosomal recessive disease characterized by progressive spondyloepimetaphyseal dysplasia and mild to severe mental retardation. MATERIAL AND METHODS: We report an uncommon association of two siblings with Dyggve-Melchior-Clausen syndrome, odontoid hypoplasia and atlantoaxial instability. Both the patients were treated with Goel?s atlantoaxial fixation procedure. RESULTS: The patients had a remarkable neurological recovery following the stabilization procedure. CONCLUSION: Atlantoaxial instability is a potentially life-threatening condition in patients with this syndrome and should be treated early with atlantoaxial stabilization. Recognition and treatment of atlantoaxial instability in patients with Dyggve-Melchior- Clausen syndrome can give gratifying results.
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